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Minimally invasive nephrectomy is performed routinely for adult renal tumors and for many benign pediatric conditions. Although open radical nephroureterectomy remains the standard of care for Wilms tumor and most pediatric renal malignancies, there are an increasing number of reports of minimally invasive surgery (MIS) for those operations as well. The APSA Cancer Committee performed a systematic review to better understand the risks and benefits of MIS in pediatric patients with renal tumors. Methods The search focused on MIS for renal tumors in children and followed the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) checklist. The initial database search identified 491 published articles, and after progressive review of abstracts and full-length articles, 19 were included in this review. Results There were two direct comparison studies where open surgery and MIS were compared. The remaining studies reported only on minimally invasive nephrectomy. Across all studies, there were a total of 151 patients, 126 of which had Wilms tumor and 10 patients had RCC. 104 patients had MIS, with 47 patients having open surgery. In the two studies in which open surgery and MIS were directly compared, more lymph nodes were harvested during open surgery (median = 2 (MIS) vs 5 (open); mean = 2.47 (MIS) vs 3.8 (open)). Many noncomparison studies reported the harvest of 2 of fewer lymph nodes for Wilms tumor. Several MIS patients were also noted to have intraoperative spill or positive margins. Survival between groups was similar. Conclusions There is a lack of evidence to support MIS for pediatric renal tumors. This review demonstrates that lymph node harvest has been inadequate for MIS pediatric nephrectomy and there appears to be an increased risk for intraoperative spill. Survival data are similar between groups, but follow-up times were inconsistent and patient selection was clearly biased, with only small tumors being selected for MIS. Type of study Review article. Level of evidence III.

Background Minimally invasive surgery has broad applicability to pediatric diseases, including pediatric cancer resection. Neuroblastic tumors of childhood are highly variable in presentation, and so careful selection of appropriate candidates for minimally invasive resection is paramount to achieving safe and durable surgical and oncological outcomes. Methods The American Pediatric Surgical Association Cancer Committee developed questions seeking to better define the role of minimally invasive surgery for neuroblastic tumors. A search using PubMed, Medline, Embase, Web of Science, ProQuest Dissertations, and Clinical Trials was performed for articles published from 1998 to 2018 in accordance with the Preferred Reporting Items for Systematic Review and Meta-Analysis Protocols (PRISMA-P) guidelines. Results The evidence identified is all retrospective in nature. Minimally invasive surgical resection of neuroblastic tumors is safe for carefully selected smaller (4-6 cm) image defined risk factor (IDRF)-negative abdominal tumors when oncologic principles are followed. Size is a less-well defined criterion for thoracic neuroblastic tumors. Open approaches for both abdominal and thoracic tumors may be preferable in the presence of IDRF's. Conclusion Small tumors without IDRF's are reasonable candidates for minimally invasive resection. Surgical oncologic guidelines should be closely followed. The quality of data supporting this systematic review is poor and highlights the need for refinement in the study of such surgical techniques to improve knowledge and outcomes for patients with neuroblastic tumors. Type of Study Systematic Review. Level of Evidence Level III and Level IV.

Pulmonary nodules that are deep within lung parenchyma and/or small in size can be challenging to localize for biopsy. This study describes current trends in performance of image-guided localization techniques for pulmonary nodules in pediatric patients.A retrospective review was performed on patients 21 years of age undergoing localization of pulmonary nodules at 15 institutions. Localization and resection success, time in interventional radiology (IR), operating room (OR) and total anesthesia time, complications, and technical problems were compared between techniques.225 patients were included with an average of 1.3 lesions (range 1-5). Median nodule size and depth were 4 mm (range 0-30) and 5.4 mm (0-61), respectively. The most common localization techniques were: wire + methylene blue dye (MBD) (28%), MBD only (25%), wire only (14%), technetium-99 only (11%), coil + MBD (7%) and coil only (5%). Localization technique was associated with institution (p 0.01); technique and institution were significantly associated with mean IR, OR, and anesthesia time (all p 0.05). Comparing techniques, there was no difference in successful IR localization (range 92-100%, p = 0.75), successful resection (94-100%, p = 0.98), IR technical problems (p = 0.22), or operative complications (p = 0.16).Many IR localization techniques for small pulmonary nodules in children can be successful, but there is wide variability in application by institution and in procedure time.Retrospective review, Level 3.

This article reviews of the current evidence-based treatment standards for children with Wilms tumor. In this article, a summary of recently completed clinical trials by the Children’s Oncology Group are provided, the current diagnostic evaluation and surgical standards are discussed, and the surgical impact on current risk stratification for patients with Wilms tumor is highlighted. LEVEL OF EVIDENCE: This is a review article of previously published and referenced LEVEL 1 studies, but also includes expert opinion LEVEL V, represented by the American Pediatric Surgical Association Cancer Committee.

Background/Purpose Thoracoscopic excision of pulmonary nodules is often required for diagnostic or therapeutic purposes, however subpleural and sub-centimeter nodules can be difficult to visualize. Various CT-guided localization techniques have been described, though there is minimal published pediatric data regarding the use of microcoils. We hypothesize that microcoil localization facilitates thoracoscopic resection of pulmonary nodules in children. Methods A multi-institutional retrospective review of children who underwent preoperative CT-guided localization of lung nodules was conducted from 2012 to 2019. A combination of methylene blue dye (MBD), wires, and microcoils were utilized for CT-guided localization. When microcoils were utilized, fluoroscopy assisted in lesion identification and resection. Results Eighteen patients (mean age 13 years, range 2–21 years) underwent thoracoscopic resection of 24 preoperatively localized pulmonary nodules. Mean size and depth of the lesions were 5.5 mm and 10 mm, respectively. Microcoil placement was successful 95% of the time and assisted in lesion localization in 88% of cases. Wire localization was not a durable technique, as 3 of 5 wires became dislodged upon lung isolation. Conclusions Preoperative CT-guided localization with microcoils can assist in fluoroscopic-guided resection of pulmonary nodules in children. This technique avoids the pitfall of wire dislodgement, and provides surgeons an additional technique to localize sub-centimeter, subpleural nodules. Type of Study Retrospective Review. Level of Evidence Level III.

Objective To perform a multicenter review of outcomes in patients with H-type tracheoesophageal fistula (TEF) in order to better understand the incidence and causes of post-operative complications. Background H-type TEF without esophageal atresia (EA) is a rare anomaly with a fundamentally different management algorithm than the more common types of EA/TEF. Outcomes after surgical treatment of H-type TEF are largely unknown, but many authoritative textbooks describe a high incidence of respiratory complications. Methods A multicenter retrospective review of all H-type TEF patients treated at 14 tertiary children's hospital from 2002–2012 was performed. Data were systematically collected concerning associated anomalies, operative techniques, hospital course, and short and long-term outcomes. Descriptive analyses were performed. Results We identified 102 patients (median 9.5 per center, range 1–16) with H-type TEF. The overall survival was 97%. Most patients were repaired via the cervical approach (96%). The in-hospital complication rate, excluding vocal cord issues, was 16%; this included an 8% post-operative leak rate. Twenty-two percent failed initial extubation after repair. A total of 22% of the entire group had vocal cord abnormalities (paralysis or paresis) on laryngoscopy that were likely because of recurrent laryngeal nerve injury. Nine percent required a tracheostomy. Only 3% had a recurrent fistula, all of which were treated with reoperation. Conclusions There is a high rate of recurrent laryngeal nerve injury after H-type TEF repair. This underscores the need for meticulous surgical technique at the initial repair and suggests that early vocal cord evaluation should be performed for any post-operative respiratory difficulty. Routine evaluation of vocal cord function after H-type TEF repair should be considered. The level of evidence rating Level IV.

Background Differentiated thyroid carcinomas (DTCs) are rare in young children but represent almost 10% of all malignancies diagnosed in older adolescents. Methods This article reviews the recent literature describing surgical therapeutic approaches to pediatric DTC, associated complications, and long-term recurrence and survival outcomes. Results Similar to adult thyroid cancers, pediatric DTCs are more common in females and are associated with thyroid nodules, family history of thyroid cancer, radiation exposure, iodine deficiency, autoimmune thyroid disease, and genetic syndromes. Management of thyroid cancers in children involves ultrasound imaging, fine needle aspiration, and surgical resection with treatment decisions based on clinical and radiological features, cytology and risk assessment. Conclusions Total thyroidectomy and compartment based resection of clinically involved lymph node basins form the cornerstone of treatment of DTC. There is an evolving literature regarding the use of molecular genetics to inform treatment strategies and the use of targeted therapies to treat iodine refractory and surgically unresectable progressive disease. Type of study Summary review. Level of evidence This is a review article of previously published Level 1–5 articles that includes expert opinion (Level 5).

Elective laparoscopic cholecystectomy (LC) pediatric patients in our institution have historically been admitted for an overnight hospital stay (OHS). The purpose of this study was to implement an ERAS protocol for elective LC in pediatric patients to promote same-day discharge (SDD) while maintaining excellent outcomes.An ERAS protocol for elective LC was implemented encompassing pre-, peri-, and postoperative management. A retrospective review of prospectively collected data from patients before (BI) and after implementation (AI) of the protocol was performed.A total of 250 patients (BI 105, AI 145) were included in the study. The AI group had significantly higher rate of SDD compared to BI (77.2% vs. 1.9%, p 0.01) and significantly decreased opioid use (morphine equivalents mg/kg AI 0.36 vs. BI 0.46, p 0.001). There were also no significant differences in the rate of total 30-day emergency department visits (BI 11.4% vs. AI 9.7%, p = 0.52) or surgery-related 30-day emergency department visits (BI 7.6% vs. AI 8.3%, p = 0.53). Factors that predisposed patients to an OHS after LC included higher ASA, later surgery start times, and longer operative times.The ERAS protocol significantly increased the rate of SDD after elective LC in pediatric patients without an associated increase in emergency department visits or readmissions.III.

Background/Purpose Melanoma is the most common skin cancer in children and often presents in an atypical fashion when compared to adults. The purpose of this review is to present an update on the epidemiology, surgical and medical management and prevention strategies in pediatric melanoma. Methods A comprehensive review of the current literature on the epidemiology, surgical and medical management and prevention of adult and pediatric melanoma was performed by the authors and the results of this review are summarized in the manuscript. Results Most recently, the incidence of melanoma in children has been declining, possibly owing to increased awareness and sun exposure prevention. The mainstay of therapy is surgical resection, often with sentinel lymph node biopsy. A positive sentinel node has prognostic value; however, completion node dissection is no longer recommended in the absence of clinically or radiographically positive nodes. Those with advanced disease also receive adjuvant systemic therapy using increasingly targeted immunologic therapies. Conclusions Sentinel lymph node positive patients no longer require completion lymph node dissection and instead may be followed by ultrasound. However, it is important to note that children have been excluded from most melanoma clinical trials to date, and therefore, recommendations for management are based on existing pediatric retrospective data and extrapolation from adult studies. Level of evidence IV.

Transanastomatic feeding tube (TAT) use in the repair of tracheoesophageal fistulas (TEF) with or without esophageal atresia (EA) and EA with or without TEF allows for earlier enteral feedings, however, may predispose to esophageal stricture.We review our institutional experience with esophageal atresia repair over a 15-year period from 2000 to 2015 and report on our observed complication rate with emphasis on the surgical approach and use of TATs.We identified 110 TEF repairs. Ninety-six were Type C, 7 were Type A, 4 were Type D, and 3 were Type E (H-Type). TATs were used in 74% of patients. The stricture rate with the TAT approached 56% versus 17% without a TAT (p0.0005). There was no difference in leak rate (p=0.27). Ninety-four TEF repairs were performed via open thoracotomy, and 16 were initially approached thoracoscopically. Six out of 16 that were started thoracoscopically were completed with the minimally invasive approach. Whether the case was started thoracoscopically, completed thoracoscopically, or performed open made no difference in the rate of stricture or anastomotic leak, but we did observe an increase in musculoskeletal complications in the open thoracotomy group (28% vs. 0).Our data suggests that the use of TATs does not protect against anastomotic leak, but may increase stricture rate. Further, the thoracoscopic group showed no difference in the leak or stricture rate and demonstrated less musculoskeletal complications. Confirmation of these findings will require a prospective study.III.

Neuroblastoma is an embryonic cancer arising from neural crest stem cells. This cancer is the most common malignancy in infants and the most common extracranial solid tumor in children. The clinical course may be highly variable with the possibility of spontaneous regression in the youngest patients and increased risk of aggressive disease in older children. Clinical heterogeneity is a consequence of the diverse biologic characteristics that determine patient risk and survival. This review will focus on current progress in neuroblastoma staging, risk stratification, and treatment strategies based on advancing knowledge in tumor biology and genetic characterization. TYPE OF STUDY: Review article. LEVEL OF EVIDENCE: Level II.

Purpose Bladder cancer is the sixth most common cancer in the United States, but is exceedingly rare in young patients, leading to a lack of accepted standards for diagnosis, treatment, and surveillance. We review our institutional experience with bladder urothelial neoplasms in pediatric and young adult patients summarizing presentation, treatment, and outcomes. Methods Surgical pathology records at our institution were searched for cases of urothelial neoplasms among patients ≤25 years of age treated between January 1997 and September 2016. Cases submitted exclusively for pathology review were excluded. Diagnoses were confirmed based on pathologic examination using the 2004 World Health Organization classification system. Results Thirty-four patients were identified with a mean age of 21.1 years (range 8–25 years), and median follow-up was 25.1 months (1–187 months). The male to female ratio was 1.83:1. The most common presenting symptom was hematuria (n=26; 76%). Diagnoses were invasive urothelial carcinoma (n=3), noninvasive urothelial carcinoma (n=24), PUNLMP (n=6), and urothelial papilloma (n=1). Noninvasive lesions were resected by cystoscopy, after which 12% (n=4) experienced complications (grade II or greater). One patient with stage IV invasive disease at diagnosis died, and 2 patients developed recurrences. Of those with noninvasive carcinoma, 29% (n=7) required repeat cystoscopy soon after initial TURBT at outside institutions, and 17% (n=4) had tumors downgraded from high-grade to low-grade after pathology review. Conclusion Hematuria is the most common sign of bladder neoplasia in children and young adults and should be investigated by cystoscopy. The majority of urothelial neoplasms in these patients are noninvasive and can be successfully treated with transurethral resection. Level of evidence Level IV (Retrospective study with no comparison group).

Background Lymphatic mapping to guide sentinel lymph node biopsy (SLNB) typically requires lymphoscintigraphy prior to surgery. In young pediatric patients, this process often requires intubation in the nuclear medicine suite followed by transport to the operating room (OR). Methods We reviewed 14 pediatric cases in which a portable nuclear imaging camera was utilized to perform the entirety of the SLNB in the OR. Results and Conclusion This method, utilizing intraoperative nuclear imaging, helped to confirm removal of the sentinel lymph node in real time, decreased anesthesia time, and avoided transport of a sedated or intubated child. Level of Evidence III.

Background Bilateral rectus sheath blocks have proven to be superior to local anesthetic infiltration for umbilical incisions and have been gaining popularity for the treatment of perioperative pain in children. We aim to develop a technique of surgeon performed rectus sheath blocks under laparoscopic-guidance alone. Methods In phase I, we observed the laparoscopic appearance of a rectus sheath block. The pain management team performed an ultrasound-guided rectus sheath nerve block as we visualized the posterior rectus sheath with the laparoscope. In phase II, after completion of the laparoscopic procedure, we performed a rectus sheath nerve block. Ultrasound was used to identify where the local anesthetic had been injected. Results Nineteen patients were included in this study, accounting for 38 rectus sheath blocks. In phase I, we observed with the laparoscope the delivery of ten ultrasound-guided rectus sheath blocks. In phase II, 28 laparoscopic-guided rectus sheath blocks were completed with immediate ultrasound confirmation of correct placement. Conclusions We have demonstrated that the rectus sheath nerve block can be performed reliably under laparoscopic-guidance alone. The efficacy of the laparoscopic-guided nerve block compared to the ultrasound-guided approach will need further study in a prospective, randomized trial. Type of study Retrospective review. Level of evidence 3.

Background/purpose Fibrolamellar hepatocellular carcinoma (FL-HCC) arises in pediatric/adolescent patients without cirrhosis. We retrospectively evaluated the impact of resection, nodal status, metastasis, and PRETEXT stage on overall survival (OS). Methods With IRB approval, we reviewed records of 25 consecutive pediatric patients with FL-HCC treated at our institution from 1981 to 2011. We evaluated associations between OS and PRETEXT stage, nodal involvement, metastasis, and complete resection. Results Median age at diagnosis was 17.1years (range, 11.6–20.5). Median follow-up was 2.74years (range, 5–9.5). Five (28%) patients had PRETEXT stage 1 disease, 10 (56%) had stage 2, 2 (11%) had stage 3, and 2 (11%) had stage 4 disease. On presentation, 17 (68%) patients had N1 disease, and 7 (28%) had parenchymal metastases. Complete resection was achieved in 17 (80.9%) of 21 patients who underwent resection. Five-year OS was 42.6%. Survival was positively associated with complete resection ( P =0.003), negative regional lymph nodes ( P =0.044), and lower PRETEXT stage ( P P =0.05). Conclusions In young patients with FL-HCC, lower PRETEXT stage and complete resection correlated with prolonged survival, while metastatic disease and positive lymph node status were associated with poor prognosis. Thus, we recommend complete resection and regional lymphadenectomy whenever possible.

Purpose: Neuroblastoma is the most common extracranial solid tumor in children. Twenty percent of all neuroblastomas arise in the thorax. This study evaluates the open vs thoracoscopic resection of thoracic neuroblastoma. Methods: A retrospective chart review was conducted from the medical records of all children undergoing resection of a thoracic neuroblastoma from 1990 to 2007 at our institution. We evaluated patients who underwent open vs thoracoscopic resection and compared demographics, pathologic condition, stage, operative details, complications, and outcomes between the 2 groups. Results: A total of 149 cases of neuroblastoma were identified during the study period, 36 (24%) of which had tumor located in the thorax. Thirty-six of these patients underwent 37 operations for primary thoracic neuroblastoma. Open thoracotomy was used in 26 cases with the thoracoscopic approach to resection used in the remaining 11. We observed no differences in patient demographics including mean age, sex, or ethnicity. Tumors in both groups were of similar histologic condition, location, surgical margin, lymph node status, and stage. The length of operation was similar between the 2 groups, but length of stay was shorter in the thoracoscopic group (2.0 days; range, 1-7 days vs 3.5 days; range, 2-8 for the open group; P = .01). Estimated blood loss was also less in the minimally invasive group (median, 10 mL; range, 0-75 mL vs 25 mL; 5-650 mL in the open group; P = .02). Review of outcomes showed no significant difference in complications, recurrence, survival, or disease-free survival between these 2 groups. Conclusions: This retrospective review of thoracic neuroblastoma for an 18-year period shows that thoracoscopic resection is an effective approach to this tumor and offers shorter length of stay and decreased blood loss when compared to open thoracotomy.

Because malrotation most commonly presents in infants, treatment recommendations for older children (1 year) have been based on data obtained from small case series. The purpose of this study was to use a large national database to determine the clinical significance of older children presenting with malrotation to develop treatment recommendations for this group.Records of children undergoing a Ladd's procedure were identified in the Kids' Inpatient Database, an administrative database that contains all pediatric discharges from 27 states during 2000. Patient characteristics, associated diagnoses, operations performed, and mortality were evaluated. Discharge weighting was used to obtain a national estimate of the number of children older than 1 year treated for malrotation.Two hundred nineteen older children (1 and18 years) undergoing a Ladd's procedure were identified in the database. One hundred sixty-four (75%) of these patients were admitted for treatment of malrotation, whereas most of the remaining 55 patients (25%) were admitted for another diagnosis and underwent a Ladd's procedure incidental to another abdominal operation. Seventy-five patients underwent a Ladd's procedure during an emergency admission. Thirty-one patients had volvulus or intestinal ischemia, 7 underwent intestinal resection, and 1 patient died. Based on case weightings, it was estimated that 362 older children underwent a Ladd's procedure for symptoms related to malrotation in 2000 in the United States (5.3 cases per million population).These findings provide support for performing a Ladd's procedure in older children with incidentally found malrotation to prevent the rare but potentially devastating complications of this anomaly.